The principal functional elements of the nervous system are nerve cells or neurons that make up between 10% and 15% of the total number of cell elements in the nervous system. The remaining, greater part of the nervous system is taken up by neuroglial cells.
The function of the neurons consists in receiving signals from receptors or other nerve cells, storing and processing the information received, and sending nerve impulses to other cells—nerve, muscle, or secretory. The glial elements constituting the bulk of the nervous tissue fulfill auxiliary functions and fill up almost the entire space between the neurons. In anatomical terms, they are distinguished into neuroglial cells in the brain (oligodendrocytes and astrocytes) and Schwann cells in the peripheral nervous system. Oligodendrocytes and Schwann cells form myelin sheaths around axons (extensions of nerve cells).
Myelin is a specific kind of cell membrane surrounding the extensions of nerve cells, most of them axons, in the central and peripheral nervous systems. In chemical composition, myelin is a lipoprotein membrane consisting of a biomolecular lipid layer that lies between the monomolecular layers of protein and is spirally wrapped around the internodal segment of a nerve fiber. The principal functions of myelin include metabolic isolation and acceleration of nerve impulse conduction, along with supporting and barrier functions.
Diseases, one of the principal manifestations of which are destruction of nerve fibers and destruction of myelin, are today one of the formidable challenges facing clinical medicine, neurology above all. There has been a recognizable increase in recent years in the number of cases attended by myelin damage.
Myelin destruction may be related to biochemical defects of its structure that are, as a general rule, genetically predetermined or result from damage caused to normally synthesized myelin under the effect of various forces.
Myelin destruction is a general mechanism of the nerve tissue reacting to any damage caused to it. Nervous diseases related to myelin destruction may be divided into two main groups—myelopathies and myeloclasties. An overwhelming majority of myelopathies is related to hereditary diseases that result in genetically caused biochemical defects of myelin structure. The underlying cause of myeloclastic diseases is destruction of normally synthesized myelin under the effect of various forces, both external and internal. Division of the diseases dealt with here into these two groups is very tentative because early manifestations of myelopathies may be explained by the effect of various external factors, while myeloclasties develop, most probably, in persons who are predisposed to them.
An example of hereditary myelopathies is provided by adrenoleukodystrophies (ALD) that are related to adrenocortical insufficiency and are distinguished by active diffusive demyelination of various part of both the central and peripheral nervous systems.
The principal metabolic defect caused by this disease is the rising content of long-chain saturated fatty acids (particularly, C-260) that causes serious disorders in the structure and functions of myelin. Clinical manifestations include growing weakness in the legs, disorder of polyneurotic type sensitivity (“sock” and “gloves”), and coordination disorders. An efficient specific ALD treatment does not exist today, and, therefore, symptomatic therapy is used instead.
A late form of Merzbacher-Pelizaeus sudanophilic leukodystrophy, with the onset of the disease in the second decade of life, has been described. The pronounced demyelinating damage to the brain of those patients is attended by a reduced content of cholesterol esters. The patients show progressing coordination disorders, spastic pareses, and intellectual disorders.
The group of leukodystrophies is distinguished by demyelination attended by diffuse fibrous degeneration of the white matter of the brain and formation of globoid cells in the brain tissue. Among them, Alexander's disease deserves a special attention, because it is a rare disease inherited predominantly in the autosomno-recessive type. This demyelination is distinguished by that galactolipides and cerebrosides are replaced with glucolipides accumulating in myelin. Its typical manifestations are growing spastic paralyses, reduction in the acuity of vision and dementia, epileptic syndrome, and hydrocephalus.
Also listed in the group of globoid-cell leukodystrophies are Krabbe's disease and Canavan's disease. These diseases rarely develop in adulthood. In clinical terms, they are distinguished by progressing damage to myelin in different parts of the central nervous system, resulting in pareses, coordination disorders, dementia, blindness, and epileptic syndrome.
Special attention among myeloclastic diseases must be given to viral infections, with myelin destruction playing a key role in their pathogenesis. These are, in the first place, neuro-AIDS caused by the human immunodeficiency virus (HIV), and damage to the nervous system, and also tropical spinal paraparesis (TSP) caused by the HTLV-I retrovirus.
Pathogenesis of primary damage to the CNS by the above viral diseases is related to the direct neurotoxic effect of the viruses and also to the pathological effect of cytotoxic T cells, antibodies, and neurotoxic substances produced by the infected immunocytes. Direct damage to the brain in the case of HIV infection results in the development of sub-acute encephalitis with demyelinated patches.
Treatment of all viral infections is based on the use of antiviral preparations inhibiting propagation of the virus in the infected cells.
People experiencing cachexia and suffering from chronic alcoholism, severe chronic diseases of the liver and kidneys, and in cases of diabetic keto-acidosis, are likely to develop, during resuscitation, a severe demyelinating disease—acute or sub-acute central pontine and/or extra-pontine myelinolysis. In this disease, symmetric bilateral demyelination centers are formed in the subcortical nodes and stem of the brain. It is held that this process evolves from an electrolyte balance disorder, Na ions, in the first place. The risk of myelinolysis is the highest in response to fast correction of hypo-sodaemia. In clinical terms, this syndrome can take the forms of either minimal neurological symptoms or severe alternating symptoms and evolution of coma. Typically, the disease ends in death within a few weeks, but, in some cases, heavy doses of corticosteroids prevent a lethal outcome.
Chemo- and radiotherapy may be followed by an onset of toxic leuko-encephalopathy and focal demyelination, combined with multi-focal necrosis. Another possibility is development of acute, early deferred, and late demyelinating processes. These last begin within a few months or years from irradiation and are distinguished by a severe progress and polymorphous focal neurological symptomatology. A significant role is played in the pathogenesis of these diseases by autoimmune reactions to myelin antibodies, damage of oligodendrocytes, and, therefore, disturbance of remyelination processes. Toxic damage to myelin can also be observed in cases of porphyria, hypothyroidism, intoxication by mercury, lead, CO, and cyanides, in all cases of cachexia, overdoses of anticonvulsants, isoniazid, and actinomycin, and in cases of heroin and morphine drug addiction.
Special attention must be focused on a series of myelinoclastic diseases that may be regarded as specific versions of disseminated sclerosis.
Concentric sclerosis, or Ballo's disease, is a steadily advancing demyelinating disease among people in a young age. This disease causes large demyelination foci to form predominantly in the white matter of forehead lobes, sometimes involving the gray matter as well. The foci consist of alternating regions of complete and partial demyelination, with a pronounced early damage to the oligodendrocytes.
It is worthwhile to note that demyelination foci in the CNS are fairly frequently detected in patients suffering from systemic lupus erythematosus, and primary Sjögren's syndrome, attended by vasculites of different genesis and other systemic autoimmune diseases. Myelin destruction and development of autoimmune reactions to its components has been observed in many vascular and paraneoplastic processes in the CNS (E. I. Gusev and A. N. Boiko, “Demyelinating Diseases of the Central Nervous System,” Consilium-Medicum, Volume 2, No. 2, 2000).
Treatment aimed at slowing down or stopping progressive development of diseases attended by demyelination is largely based on the perception of these diseases as autoimmune diseases. The autoimmune process is accompanied by the emergence of myelin-toxic antibodies and T lymphocyte killers destroying Schwann cells and myelin. The immune system is corrected by immunosuppressants reducing the activity of the immune system and immunomodulators altering the proportions of nervous system components. Immunosuppression and immunomodulation are intended to destroy, remove or modify the functions of lymphocytes capable of damaging myelin.
Among the methods affecting the autoimmune mechanisms of a disease, preference is given to plasmapheresis, intravenous injection of human IgG, and use of corticosteroids (“Neuropathy,” edited by N. M. Zhulev, St. Petersburg, 2005).
Plasmapheresis, however, can only be performed in a hospital environment, and its application is not always justified for patients who have retained ability to move unassisted.
The use of IgG is contraindicated in cases of anaphylactic responses, and cardiac and renal insufficiency. Complications have been observed in approximately 10% of the patients treated.
Corticosteroid therapy is administered taking into consideration a patient's history of common contraindications (peptic ulcers of the stomach and duodenum, high arterial pressure, diabetes, and so on), and using preparations inhibiting development of the most frequent complications (potassium preparations, ascorbic acid, rutin, and so on).
Available literature contains references to Copaxone-Teva, a preparation of non-interferon nature (its international name is glatiramer acetate). Copaxone-Teva is an acetate of synthetic polypeptides produced by four natural amino acids—L-glumatic acid, L-alanine, L-tyrosine, and L-lysine—and have similar elements with the basic protein of myelin in chemical structure. It belongs in the class of immunomodulators and is capable of blocking myelin-specific autoimmune reactions that are basic to the destruction of the myelin sheath of nerve fibers in disseminated sclerosis. Numerous side reactions (abscesses and hematomas at injection points, elevated arterial pressure, splenomegaly, allergic reactions, apaphylaxia, arthritis, headache, depression, spasms, bronchial spasms, impotence, amenorrhea, hematuria, and so on) have been observed when the preparation is used on a clinical scale (Khokhlov, A. P., and Savchenko, Y. N., “Myelinopathies and Demyelinating Diseases,” Moscow, 1991).
According to available publications, herb preparations are known to be used to prevent development of neuron demyelination, in particular, various preparations of plantain, American artichoke, chicory, dandelion, knot-grass, couch-grass, pumpkin, and immortelle, such as Polyvitachol, Polysponin, Chitochol, Chitolen. Siperpar, Tykveol, Tykveinol, and Rosoptin (Korsun, V. F., and Korsun, E. V., “Herbs to Treat Disseminated Sclerosis: A Textbook in Methodology,” INFIT, Moscow, 2004).
Also known in the art is stephaglabrin sulfate (Stephaglabrini sulfas), a sulfate of stepharine alkaloid extracted from the tubers and roots of Stephania glabra (Rob) Miers, Menispermaceae family, a perennial tropical herb growing in the subtropical and tropical mountainous areas of South China, Japan, Burma, Vietnam, and India. Attempts were—undertaken in the former U.S.S.R. to introduce the plant in the subtropics of the South Caucasus, but they ended in failure. Most of the raw material is now imported from India. Known in the art is also a method for producing stephaglabrin from plant material (U.S.S.R. Inventor's Certificate No. 315,387, 1963),
Known in the art is production of a Stephania glabra line in a suspension culture yielding a high percentage of stepharine alkaloid by synthesis. The Stephania glabra culture was obtained in vitro at the Medicinal Plants Institute (VILAR). A project to develop a selection system in vitro was undertaken at the Pharmaceutical Plants Institute (IFR).
The medicament based on stephaglabrin sulfate (a sulfate of stepharine alkaloid) (C18H19O3N2)2.H2SO4, relates to proaporphine derivatives.

The sulfate is a white crystalline powder having a melting point of 245-246° C. (in vacuum), well soluble in water and aqueous alcohol. Stephaglabrin sulfate suppresses the activity of true and false cholinesterase, has a tonic effect on smooth muscles, and lowers arterial pressure. It has a low toxicity.
In the past, stephaglabrin sulfate was authorized for use in medical practice as an anti-cholinesterase medicament (U.S.S.R. Inventor's Certificate No. 315,388, 1963).
The inventors' continued studies showed that stephaglabrin sulfate has a specific inhibiting activity in relation to connective tissue development, preventing formation of scars as a result of damage to a nerve, and may be used as a medicament to heal traumatic and post-operation injuries to the peripheral nervous system (U.S.S.R. Patent No. 1,713,151, 1985).